Familial pityriasis rubra pilaris
Last updated 18 Aug 2020
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Familial pityriasis rubra pilaris is a rare genetic condition that affects the skin. The name of the condition reflects its major features: The term "pityriasis" refers to scaling; "rubra" means redness; and "pilaris" suggests the involvement of hair follicles in this disorder. Affected individuals have a salmon-colored skin rash covered in fine scales. This rash occurs in patches all over the body, with distinct areas of unaffected skin between the patches. Affected individuals also develop bumps called follicular keratoses that occur around hair follicles. The skin on the palms of the hands and soles of the feet often becomes thick, hard, and callused, a condition known as palmoplantar keratoderma.
Description
Familial pityriasis rubra pilaris is a rare genetic condition that affects the skin. The name of the condition reflects its major features: The term "pityriasis" refers to scaling; "rubra" means redness; and "pilaris" suggests the involvement of hair follicles in this disorder. Affected individuals have a salmon-colored skin rash covered in fine scales. This rash occurs in patches all over the body, with distinct areas of unaffected skin between the patches. Affected individuals also develop bumps called follicular keratoses that occur around hair follicles. The skin on the palms of the hands and soles of the feet often becomes thick, hard, and callused, a condition known as palmoplantar keratoderma.
Researchers have distinguished six types of pityriasis rubra pilaris based on the features of the disorder and the age at which signs and symptoms appear. The familial form is usually considered part of type V, which is also known as the atypical juvenile type. People with familial pityriasis rubra pilaris typically have skin abnormalities from birth or early childhood, and these skin problems persist throughout life.
Inheritance patterns
The source lists the following possible inheritance patterns: Autosomal dominant. More than one pattern can apply in different cases.
Related genes
Other names
Familial PRP
Related patient information and medicines
Patient information leaflet
Skin rashes
There are many different types of skin rashes and many different causes of skin rashes. Although most skin rashes are harmless, some do need treatment (which may be tablets, creams or ointments) from your doctor or pharmacist. Fungal (or 'yeast') skin infection, such as athlete's foot , groin infection (tinea cruris) , ringworm, scalp ringworm or.
Patient information leaflet
Viral skin infections
Chickenpox is a common example (although you can also catch chickenpox through the air). Molluscum contagiosum and herpes simplex are probably the other most common examples we see in the UK. In the farming communi... More common examples are pityriasis rosea and hand, foot and mouth disease. (This virus can lie dormant in your body and then come.
Patient information leaflet
Pityriasis rosea
Pityriasis rosea is a self-limiting rash, In other words it goes away on its own without any treatment. Although the rash can be quite dramatic, the illness is very mild. Pityriasis rosea is a self-limiting rash that can occur both in adults and in children. Rarely, it can cause scaling or flaking of the skin, which can be troublesome... Further.
Patient information leaflet
Guttate psoriasis
Guttate psoriasis is a skin condition which gives you light red, scaly patches scattered over almost your whole body. It often happens a few weeks after a throat infection with a particular germ (bacterium) called a group A beta-haemolytic streptococcus. What is guttate psoriasis. Although it has the name 'psoriasis', guttate psoriasis is almost a.
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Bowel cancer screening
Bowel (colorectal) cancer is common. The outlook (prognosis) and chance of cure are much better if this cancer is detected at an early stage rather than at a later stage. A screening programme operates in the UK for certain age groups. These groups of people offered extra screening tests include: People with certain inherite... These are rare.
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Keratosis pilaris
Keratosis pilaris is a very common skin condition which affects children and teenagers more than adults. It causes rough skin, particularly over the upper arms, outer legs and buttocks. What is keratosis pilaris. Keratosis pilaris is a very common skin condition which causes rough skin with raised red bumps. It is caused by "keratin" - the protein.
Further reading and references
- Familial pityriasis rubra pilaris; MedlinePlus, National Library of Medicine.
- Fuchs-Telem D, Sarig O, van Steensel MA, Isakov O, Israeli S, Nousbeck J, Richard K, Winnepenninckx V, Vernooij M, Shomron N, Uitto J, Fleckman P, Richard G, Sprecher E. Familial pityriasis rubra pilaris is caused by mutations in CARD14. Am J Hum Genet. 2012 Jul 13;91(1):163-70. doi: 10.1016/j.ajhg.2012.05.010. Epub 2012 Jun 14.
- Klein A, Landthaler M, Karrer S. Pityriasis rubra pilaris: a review of diagnosis and treatment. Am J Clin Dermatol. 2010;11(3):157-70. doi: 10.2165/11530070-000000000-00000.
- Sehgal VN, Srivastava G. (Juvenile) Pityriasis rubra pilaris. Int J Dermatol. 2006 Apr;45(4):438-46. doi: 10.1111/j.1365-4632.2006.02666.x. No abstract available.
- Thomson MA, Moss C. Pityriasis rubra pilaris in a mother and two daughters. Br J Dermatol. 2007 Jul;157(1):202-4. doi: 10.1111/j.1365-2133.2007.07938.x. Epub 2007 May 8. No abstract available.
- Vanderhooft SL, Francis JS, Holbrook KA, Dale BA, Fleckman P. Familial pityriasis rubra pilaris. Arch Dermatol. 1995 Apr;131(4):448-53.
- Vasher M, Smithberger E, Lien MH, Fenske NA. Familial pityriasis rubra pilaris: report of a family and therapeutic response to etanercept. J Drugs Dermatol. 2010 Jul;9(7):844-50.
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